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Island Singapore Flap Vaginoplasty of Two Adult Cases of Mayer– Rokitansky–Küster–Hauser Syndrome Type I

Alex Okello Wamalwa, Stanely Ominde Khainga

School of Medicine, University of Nairobi

Correspondence to: Dr. Alex Okello Wamalwa, PO Box 30197–00202, Nairobi, Kenya; email: aleoke@gmail.com

Summary

Mayer–Rokitansky–Küster–Hauser Syndrome Type I is an emotionally distressful rare condition that limits normal sexual functioning and relationships. First described in 1989 by Wee and Joseph, the neurovascular island pudendal thigh flap (Singapore flap) has been variously utilized and modified for use in vaginal reconstruction. We report two adult cases of vaginal agenesis who underwent vaginal reconstruction using the Singapore flap. Both patients reported improved sexual lifestyle.

Key words: Island Singapore flap, Vaginoplasty, Mayer– Rokitansky–Küster–Hauser syndrome Type I

Ann Afr Surg. 2019; 16(1):40–42

DOI: http://dx.doi.org/10.4314/aas.v16i1.10

Funding: None

© 2019 Author. This work is licensed under the Creative Commons Attribution 4.0 International License.

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Introduction

Vaginal agenesis can present as isolated Müllerian aplasia or Mayer–Rokitansky–Küster–Hauser syndrome type I with an incidence of 1 per 4,500–5,000 females (1). This embryologic underdevelopment of the Müllerian duct results in isolated absence of the uterus and the proximal two-thirds of the vagina (2). The normal hormonal profile and normal secondary sexual development found in such patients is due to different embryologic origin of the ovaries. Despite the obvious primary infertility it confers, vaginal agenesis has significant psychological impact on the patient due to inability to experience a satisfactory sexual lifestyle. The Singapore flap vaginoplasty provides a sensate and pliable (thin and elastic) neo-vagina that enables sexual functioning (3). We present two adult cases of Mayer– Rokitansky–Küster–Hauser syndrome type I that underwent vaginoplasty using the island Singapore flap: the first such report in Kenya.

 

Case Presentation

We present two cases; a 27-year-old female who presented to us accompanied by her sister in November 2016, and a 30-year-old female accompanied by her spouse presenting in September 2017. The patients had similar clinical presentation. Chief complaints were primary amenorrhea and inability to experience sexual intercourse due to shortened vagina. Physical examination revealed normal secondary sexual characteristics, blind ending distal vaginal canal of 4 cm and an absent cervix, in both patients. An absent uterus with normal bilateral ovaries was reported on ultrasonography. Hormonal profile was normal (luteinizing hormone, follicle-stimulating hormone, prolactin, estradiol and p